Hypertrophic pachymeningitis (HP) is a rare form of diffuse inflammatory disease that causes thickening the dura mater. It can involve cranial or spinal both. An increasingly well-known symptom IgG4-related illness, fibroinflammatory syndrome may affect almost any organ, hypertrophic (IgG4-RHP). estimated IgG4-RHP account for high proportion cases once considered idiopathic. Contrast magnetic r...

IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world. The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4(+) plasma cells. Although the clinical manifestations in various organs have been established, the pathogenesis of IgG4-RD is still unknown. R...

IgG4-related disease (IgG4-RD) is characterized by elevated serum IgG4 and marked infiltration of IgG4-positive cells in multiple organs. Interleukin-33 (IL-33) is a recently described cytokine that is secreted by damaged epithelial cells, macrophages, and dendritic cells, and potently activates helper T type 2 (Th2) immune responses, which have been suggested to play a major role in IgG4 produ...

Recently, great attention has been drawn to IgG4-related diseases such as autoimmune pancreatitis (AIP) sclerosing sialadenitis, retroperitoneum fibrosis, sclerosing cholangitis. IgG4-related diseases are characterized by high serum IgG4 concentrations, sclerosing inflammation with numerous IgG4-positive plasma cells, and steroid sensitivity irrespective of their organs of origin. In this repor...

Human IgG4 antibody shows therapeutically useful properties compared with the IgG1, IgG2, and IgG3 subclasses. Thus IgG4 does not activate complement and shows conformational variability. These properties are attributable to its hinge region, which is the shortest of the four IgG subclasses. Using high throughput scattering methods, we studied the solution structure of wild-type IgG4(Ser(222)) ...

BACKGROUND IgG4-related kidney disease is a comprehensive term for renal lesions associated with IgG4-related disease, which mainly manifests as plasma cell-rich tubulointerstitial nephritis with increased IgG4+ plasma cells and fibrosis. IgG4-related kidney disease in the renal pelvis is rare. CASE PRESENTATION We describe a 53-year-old Asian woman who was referred to our hospital with a spa...

BACKGROUND/AIMS Few studies have differentiated immunoglobulin G (IgG) 4-related sclerosing cholangitis (IgG4-SC) from hilar cholangiocarcinoma (CC). Thus, we sought to investigate useful features for differentiating IgG4-SC from hilar CC. METHODS We retrospectively compared clinical, serological, imaging, and histological features of six patients with IgG4-SC and 42 patients with hilar CC. ...

BACKGROUND Measurement of serum IgG4 had been suggested to distinguish the unique subtypes of autoimmune thyroid disease (AITD) which demonstrated patterns of fluctuating between hyperthyroidism and hypothyroidism. However, the clinical utility of serum IgG4 measurement is inconclusive due to few studies having addressed these unusual patients compared with the specificity of serum IgG4 in heal...

OBJECTIVES Immunoglobulin G4-related disease (IgG4-RD) is a multiorgan condition manifesting itself in different forms. This study aimed to investigate protein expression profiles and to find the possible biomarker for IgG4-RD by liquid chromatography mass spectrometry (LC-MS) using tissue sections in IgG4-RD patients. METHODS Protein expression profiles in five IgG4-related pancreatitis and ...

OBJECTIVES/HYPOTHESIS Immunoglobulin (Ig)G4-related disease is a systemic syndrome, characterized by sclerosing lesions that mainly affect the exocrine tissue. Although some patients with IgG4-related disease complain of nasal symptoms, there are few reports concerning the nasal manifestations of this disease. We investigated the clinical and pathological features of the nasal manifestations of...